Lymphocyte subset proportions in Guillain-Barré syndrome patients treated with plasmapheresis.

We examined the changes in the proportions of certain T cell subsets in 18 Guillain-Barré syndrome patients treated with plasmapheresis (PP). PP was performed within 8 days after the onset using a continuous-flow blood cell separator or immunoadsorption. The proportions of CD3+, CD4+ and CD4+CD45RA+ cells at the onset were decreased and that of CD19+ was increased compared with those of 25 normal controls. However, the proportions of CD4+CD45R-, CD4+HLADR+, CD4+CD25+ cells and the CD8+ subpopulations were similar in patients and controls. After PP, the altered subsets tended to normalize, particularly in the group that improved after PP.

[An autopsy case of juvenile neuronal ceroid-lipofuscinosis with dilated cardiomyopathy].

We reported an autopsy case of neuronal ceroid-lipofuscinosis (NCL3) with dilatated cardiomyopathy. A 29-year-old male patient first noticed night-blindness at the age of four years. He was pointed out retinitis pigmentosa at the age of six years and developed ataxia, mental retardation, epilepsy and myoclonus, thereafter. T1 weighted MRI showed diffuse atrophy of the cerebellum, brainstem, and cerebrum, and dilatation of the ventricular system and T2-weighted MRI showed mild high signal intensity in the white matter around the trigones of the lateral ventricles. Autopsy findings showed an abundant accumulation of ceroid-lipofuscin-like lipopigments in most neurons in the central nervous system, and curvilinear bodies and lipofuscin like granules were confirmed by electron microscopy. The heart muscle showed an increase in the accumulation of ceroid-lipofuscin-like lipopigments, severe fibrosis and fatty infiltration in the myocardium. The peculiar point of this case is NCL3 with dilated cardiomyopathy.

Impaired interleukin-2 response of mononuclear cells in Guillain-Barré syndrome.

We studied the production of and response to interleukin-2 (IL-2) by cultured peripheral blood mononuclear cells (PBMC) and the soluble IL-2 receptor levels in 10 patients with GBS (eight men and two women) aged 23-67 years. The first blood sample was obtained within seven days of the onset of neuropathic symptoms, and blood samples were collected serially after treatment with plasma exchange. PBMC from GBS patients were found to produce little IL-2 and to have a low response to IL-2 soon after the onset, but marked improvement was seen within one month, concomitantly with improvement of neuropathic symptoms. Serum soluble IL-2 receptor levels were increased at the onset, but gradually decreased thereafter. Our findings suggest that primary immune response in acute-stage GBS may be downregulated via a decrease in the function of PBMC that had previously been activated by interacting with the antigen.

Giant negative T waves in Guillain-Barré syndrome.

A Guillain-Barré syndrome patient showed giant negative T waves on electrocardiography at the height of the disease, with large left ventricular hypokinesis on echocardiography and extensive defects on 123I-meta-iodobenzylguanidine myocardial scintigraphy. Gamma-globulin improved the neurological symptoms, and the above abnormalities resolved. We speculate that cardiac sympathetic nerve endings were transiently damaged, with consequent myocardial injury, due to norepinephrine toxicity.

Multiple cerebral infarctions associated with ovarian hyperstimulation syndrome.

A 26-year-old woman on human menopausal gonadotrophin-human chorionic gonadotrophin therapy for sterility showed multiple cerebral infarctions associated with ovarian hyperstimulation syndrome. A hypercoagulable state (hemoconcentration, increased plasma levels of D-dimer and thrombin-antithrombin III complex, and decreased protein S activity) was associated with her thromboembolic events. Cerebral infarction associated with mild neurologic deficits may be overlooked in patients with ovarian hyperstimulation syndrome.

Magnetic resonance imaging and 11C-N-methylspiperone/positron emission tomography studies in a patient with the interval form of carbon monoxide poisoning.

Magnetic resonance (MR) and (11)C-N-methylspiperone ((11)C-NMSP)/positron emission tomography (PET) imagings were repeatedly performed in a 50-year-old man with the interval form of carbon monoxide (CO) poisoning. In MR images obtained when delayed neuropsychiatric symptoms developed (two months after poisoning), the inner segments of the bilateral globus pallidus appeared as high signal intensities in the T1-weighted and low signal intensities in the T2-weighted images, suggesting prior focal hemorrhage in these areas. A PET study with (11)C-NMSP performed at that time showed an increase in dopamine D2 receptor binding in the caudate and putamen. Treatment with bromocriptine was very effective and five months after the poisoning, MR and (11)C-NMSP/PET images showed improvement, concomitantly with the disappearance of the neuropsychiatric symptoms.

[A case of autosomal dominant, pure form spastic paraplegia with thinning of the corpus callosum].

Autosomal dominant hereditary spastic paraplegia (HSP) is genetically classified into three types, all of which are characterized by insidiously progressive spasticity of the lower extremities. Patients with a complicated form of autosomal recessive HSP associated with hypoplasia of the corpus callosum have been reported by Iwabuchi et al. Here we report a 64-year-old patient with a pure form of autosomal dominant HSP with thinning of the corpus callosum. He had been well until 12 years of age, when spasticity and weakness of the lower extremities began to develop. His symptoms gradually worsened and he had difficulty in walking at the age of 44. When he was 56 years old, he visited our hospital. Eleven family members over five generations have been affected, and anticipation, i.e., an apparent decrease in age of onset, has been observed. On admission, he had mild cataracts, equinovarus and pes cavus, and neurological examination revealed spastic paraplegia. However, the intelligence test was normal, and nystagmus, ataxia of the extremities, involuntary movement, orthostatic hypotension or urinary disturbance was not observed. Trinucleotide repeat diseases, such as Huntington's disease, spinocerebellar ataxia type 1, spinocerebellar ataxia type 2, Machado-Joseph disease and dentatorubral-pallidoluysian atrophy, were excluded by DNA analysis. Brain MRI at the age of 64 revealed marked thinning of the corpus callosum. We considered this patient had a pure form of HSP. However, thinning of the corpus callosum has never been reported in autosomal dominant HSP.

Fluid attenuation inversion recovery (FLAIR) images of dentatorubropallidoluysian atrophy: case report.

The white matter lesions in a patient with late adult onset dentatorubropallidoluysian atrophy (DRPLA) were studied in detail by MRI using the fluid attenuation inversion recovery (FLAIR) technique. The patient was a 60 year old woman with a family history of DRPLA, in whom the number of CAG repeats in the DRPLA gene on chromosome 12 was expanded to 59 (normal allele 10). In addition to atrophy of the cerebral cortex, cerebellum, and pontomesencephalic tegmentum, the cerebral white matter and a part of the white matter tracts within the brainstem showed prominent high signal intensities on FLAIR images. These MR findings suggest that, in addition to the degeneration of the dentatorubral and pallidoluysian systems, the pathological process extends to the white matter in DRPLA. This could be important for differentiating DRPLA from other clinically similar diseases such as Machado-Joseph disease or Huntington's disease.

Natural killer cells in patients with Guillain-Barré syndrome.

We studied natural killer (NK) cell activity and NK cell number in peripheral blood of 20 patients with Guillain-Barré syndrome (GBS). NK cell activity obtained within 7 days after onset of neuropathic symptoms in patients with GBS was significantly decreased compared with that in 20 control subjects. NK cell activity was not well correlated with disease severity. After plasmapheresis, NK cell activity recovered to the normal range. Two-color flow cytometry showed that the NK cell subsets studied were quantitatively similar in GBS patients and controls.

The brainstem and thalamic lesions in dentatorubral-pallidoluysian atrophy: an MRI study.

We studied the frequency and characteristics of brainstem and thalamic lesions in dentatorubral-pallidoluysian atrophy using MRI. Of 15 subjects diagnosed by DNA analysis, 13 had lesions in the pontine base, nine in the midbrain, and five in the thalamus. Lesions were correlated positively with the patient's age, but not with neurologic features or numbers of CAG repeats. Patients with Machado-Joseph disease or spinocerebellar ataxia 1 did not show these characteristic lesions.

Neuroimaging on delayed postanoxic encephalopathy with lesions localized in basal ganglia.

A 59-year-old woman suffered from prolonged hypotension with myocardial infarction. Sixteen days after the episode, she showed bradykinesia, gait disturbance, and postural tremor. MRI revealed low signa intensities in the bilateral caudate nuclei and putamen on the T1-weighted image and high signal intensities on the T2-weighted image. PET with 18F-FDG revealed a severe decrease in glucose metabolism in bilateral basal ganglia. It is concluded that prolonged hypotension may induce localized delayed anoxic lesions in basal ganglia.

[A patient with spontaneous intracranial hypotension--comparison between MRI findings and meningeal pathology].

We report a 44-year-old woman with spontaneous intracranial hypotension (SIH) who developed an acute, severe, nonthrobbing headache. The headache remained more severe in the occipital region and was markedly worse with upright posture. Cerebrospinal fluid (CSF) examination revealed an opening pressure of 55 mmH2O, and the CSF contained 38 cells/mm3 and 57 mg/dl of protein. The results of other laboratory examinations were unremarkable. T1-weighted MR images (MRI) of the head revealed an extensive diffuse pachymeningeal gadolinium enhancement and bilateral subdural fluid accumulation. On the 21st hospital day, a meningeal biopsy was performed through a right parietal craniotomy. On histologic examination, the dural border cell layer demonstrated nonspecific granulation tissue with mild inflammatory changes. The remaining layers of the dura mater and the arachnoid membrane showed no obvious pathological changes. We speculated that the inflammatory changes of the dural border cell layer correspond to the zone of pachymeningeal gadolinium enhancement of the MRI. The granulation tissue of the dural border cell layer and subdural fluid accumulation may represent secondary reactive phenomena, and were suspected to have been caused by downward displacement of the brain due to decreased intracranial pressure.

[A case of myeloradiculoneuropathy associated with persistent high titers of anti-GM1 and anti-GD1b antibodies].

A 60-year-old woman was admitted to our hospital with bilateral weakness of the lower limbs. She had been suffering from spastic paraparesis of unknown origin for 12 years since she was 48. Myelopathy with the Th6 level appeared when she was 52 and underwent gradual deterioration. On admission, she showed spastic paraparesis and myelopathy with the Th6 level. A thoracic spinal MRI examination revealed a wholly thin spinal cord. EMG demonstrated that the conduction velocity of the peroneal nerve was 37.8m/sec, and F waves were not evoked on stimulation of the peroneal nerve. Although the anti-GM1 and anti-GD1b antibody titers in the serum were increased on admission, their values decreased transiently after whole plasmapheresis. There was accompanying impairment of the radiculoneuropathy and myelopathy. It is important to measure the titers of anti-ganglioside antibodies in patients with myeloradiculoneuropathy of unknown origin, and whole plasmapheresis is considered to represent a useful treatment for this type of illness.

Treatment of essential and parkinsonian tremor with nipradilol.

Nipradilol is a new type of beta-blocker which possesses nitroglycerin-like vasodilating action in addition to beta-blocking action. We investigated the efficacy and safety of nipradilol for treating tremor in 20 patients with essential tremor (ET group) and 20 patients with Parkinson's disease (PD group). All patients received nipradilol (6 mg per day) for more than 8 weeks. Improvement of tremor appeared within 2 or 4 weeks after the start of nipradilol therapy, and the efficacy rate, defined as "moderately effective" or over, was 42.5% in all 40 patients, while that defined as "slightly effective" or over was 87.5%. The efficacy rate tended to be higher in the ET group compared with the PD group. Mean blood pressure was significantly decreased from the 4th week after the start of treatment and heart rate was significantly reduced from the 2nd week of treatment. Laboratory examination showed no significant changes.

Magnetic resonance imaging of herpes simplex virus encephalitis: reversible asymmetric basal ganglia lesions.

We report a patient with herpes simplex virus type 1 encephalitis (HSE) who showed abnormal magnetic resonance imaging (MRI) signals in the basal ganglia. The lesions were asymmetric and became apparent with relapse of the neurological symptoms, but they completely disappeared, concomitantly with improvement of the illness.

Reversible magnetic resonance imaging lesions in Wilson's disease: clinical-anatomical correlation.

Described herein is a patient with Wilson's disease who had tremor as a prominent neurological manifestation. T2-weighted magnetic resonance imaging showed abnormal high signal intensities in the bilateral lenticular nuclei, thalami, and red nuclei of the midbrain. Improvement of tremor with copper chelating agents was well correlated with a decrease of the abnormal signals in the thalami and the red nuclei.

[A case of medial medullary infarction with prominent deep sensory impairment].

We report a patient with medial medullary infarction who showed deep sensory impairment as his prominent neurological manifestation. A 54-year-old man with a history of hypertension was admitted to our hospital with numbness of the bilateral upper and lower extremities, followed by dysarthria and right hemiparesis. Physical examination revealed no abnormalities except for high blood pressure. He hiccuped continuously. On neurological examination, he exhibited dysarthria, mild dysphagia and right hemiparesis without facial or lingual paresis. Sensitivity to light touch and pinprick was normal, but sensitivity to vibration and joint position was severely decreased in the bilateral upper and lower extremities, predominantly in the lower extremities and on the right side in the upper extremities. He had been treated with antiedema agents and thromboxane synthetase inhibitor. His hiccups stopped within two weeks, and his right hemiparesis gradually improved within one month. However, his deep sensory impairments remained prominent. Blood examinations disclosed positive lupus anticoagulant. MRI showed bilateral infarction at the medial portion of the upper medulla oblongata, extending to both pyramids, especially on the left. Somatosensory evoked potentials (SEP) after median nerve stimulation showed P14 and the later components with prolonged latency. No SEP were recorded after posterior tibial nerve stimulation. The latency of P14 was well correlated with the severity of deep sensory impairments in the upper extremities. Neurological manifestations of our patient are not typical of medial medullary infarction, and are informative about the functional anatomy of the deep sensory tract in the medulla oblongata. We discuss the relation of the intractable hiccups to the bilateral medial medullary lesions, and emphasize the importance of lupus anticoagulant as one of the risk factors in brainstem infarction.

Construction of a quantitative three-dimensional model for odor quality using comparative molecular field analysis (CoMFA).

A quantitative structure-activity relationship (QSAR) study of odorants was performed taking an odor as an activity. As an example, we took the 'green odor of pyrazine derivations' as an activity. Conformational analysis of the pyrazine derivatives was performed, and conformers were selected using the longest side-length of a circumscribed box (LLCB) as a criterion. Comparative molecular field analysis (CoMFA) was used to elucidate the three-dimensional (3D) structural features of the derivatives. As a result, it was found that the steric and electrostatic features of the derivations were correlated with human olfactory detection threshold values. We constructed a quantitative 3D model using the graphic views of CoMFA and partial structures of the derivatives. The prediction of human olfactory detection threshold values of other pyrazine derivatives with green odor was possible by using the 3D model. As another example, we took the 'sweet odor of compounds with various structures' as an activity. A quantitative 3D model for sweet odor was constructed in the same manner. Analysing the structural features of odorants by CoMFA and constructing 3D models for several important odor qualities would help to (i) explain or predict human olfactory detection threshold values of interesting odorants, (ii) design new odorants by suggesting the steric and electrostatic requirements, and (iii) elucidate the mechanism of odorant-receptor interaction.